Abstract
PURPOSE:
Renal manifestations of tuberous sclerosis complex (TSC) occur with a high frequency and a wide range of severity. The onset and complications of each affected organ depend on the age. This study aimed to investigate the associations between comorbidities, frequency, and size of cysts/angiomyolipomas of TSC and the patients in the different ages.
METHODS:
We performed a systematic evaluation of patients with TSC at integrated clinics for TSC. The patients were diagnosed with TSC according to Roach's clinical diagnostic criteria. All the patients underwent a systematic evaluation with a medical history review, recording of cutaneous manifestations, magnetic resonance imaging of the brain, chest computed tomography scan, and cardiac echography. Each patient was routinely evaluated with renal magnetic resonance imaging.
RESULTS:
Of the 57 patients with TSC, renal lesions or manifestations were observed in 38.5 % at preschool-age group (<6 years) that increased to 75.0, 85.7, and 100 % in school-age group (6-18 years), young adulthood group (18-30 years), and middle-aged group >30 years, respectively. Renal complications or interventions were found in 72.7 % of patients >30 years. There was a strongly positive association between the size of renal lesions and age (p < 0.001). Comorbidities of TSC were significantly correlated with pulmonary lymphangiomyomatosis (p < 0.001) and seizure remission (p = 0.018).
CONCLUSIONS:
The results indicate a positive association of progressively growing renal lesions in patients with TSC with increasing age. It is mandatory for clinicians that monitor the high-risk patients to minimize and prevent renal complications in the future.
